10 things you should know about cutaneous T cell lymphoma

It’s a rare lymphoma that starts with dry skin and a red rash. Patients could easily confuse it with benign skin conditions such as psoriasis and eczema.

But in advanced stages, cutaneous T-Cell Lymphoma (CTCL), a type of non-Hodgkin lymphoma that originates in the skin, can spread through the blood stream to lymph nodes and other organs.

CTCL is a blood cancer affecting white blood cells called T lymphocytes. Because it can be hard to recognize and difficult to understand, we put together this list of important information you might not know about CTCL.

1. There’s more than one kind of CTCL.
CTCL is actually a blanket term for any T cell lymphoma that originates in the skin. The most common type of CTCL is mycosis fungoides, which accounts for about half of all cases. It usually only affects the skin and may respond to topical treatments. The next most common form is Sézary syndrome, a more advanced, aggressive variation of CTCL that may spread to other parts of the body, especially blood or lymph nodes, possibly requiring a combination of systemic biologic and skin-directed therapies. Extracorporeal photopheresis, a technique where a small amount of white blood cells is treated with a photoactive drug that is then activated with ultraviolet light, is commonly used for Sézary syndrome patients.

2. We don’t know what causes it.
Researchers have yet to find any specific causes of CTCL. According to the National Organization for Rare Disorders, potential causes that researchers are looking at include genetic abnormalities, environmental factors, or even chronic viral infections that result in a chronically heightened or exhausted immune state.

3. CTCL is rare.
Of all non-Hodgkin lymphomas, only about 4 percent are CTCLs. According to the Leukemia & Lymphoma Society, “the overall annual age-adjusted incidence of CTCL is approximately six cases per one million.”

4. It can be hard to recognize.
Patients with CTCL, especially in the early stages, may not even realize they have lymphoma because the symptoms are almost identical to benign skin conditions like psoriasis and eczema. The first symptoms may be nothing more than dry skin with a red rash.

For people with darker complexions, the affected areas may be lighter or darker than the rest of their skin. These may later become raised, red patches called plaques that can become itchy and scaly. Later on, tumors can develop in the skin.

5. CTCL requires specialized, expert treatment.
Because CTCL is so uncommon, patients who do have it should find a cancer center with experts who specialize in diagnosing and treating non-Hodgkin lymphomas. At City of Hope, Christiane Querfeld, M.D., Ph.D., is director of the Cutaneous Lymphoma Program and one of the country’s top experts on CTCL.

6. History of skin conditions may increase CTCL risk.
Querfeld says that while she and other experts have yet to find any proven risk factors for CTCL, there is some evidence that people who have chronic skin conditions like eczema and psoriasis may be more likely to get it as a result of chronically inflamed skin that predispose lymphocytes to genetic aberrations.

7. Ethnicity may be a risk factor.
Among the different ethnic populations in America, African-Americans have higher incidences of CTCL, and they’re more likely to have aggressive forms. Caucasians have the second highest incidence rates, while populations of Asian descent have the lowest.

8. Older men get CTCL more than women.
There are almost double the cases of CTCL among men than women, and for both sexes it’s more common as they get older. The onset of CTCL usually happens when patients are in their 50s and 60s.

9. Every treatment regime is unique.
CTCL comes in so many variations and every patient is different, so there is no single effective treatment, and many patients get a combination of approaches. Topical treatments like corticosteroids and nitrogen mustard can be applied as lotions and creams.

Phototherapies use a mix of light-triggered medications and UV light exposure. And electron beam therapy can expose the entire skin surface of the skin to radiation without damaging internal organs.

10. Stem cell transplant is the only potential “cure.”
None of the currently available treatments eliminate CTCL, with the exception of an allogeneic stem cell transplantation, which also has significant risk of complications.

With a stem cell transplant, the patient gets high doses of chemotherapy that kills the cancerous and noncancerous cells in the blood. Then stem cells from a donor are used to repopulate the patient’s blood with healthy noncancerous cells, potentially ridding them of CTCL for good.